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A case of solitary plasmacytoma involving the left cerebral convexity, dura mater, falx, and sagittal sinus is reported. After undergoing subtotal excision, followed by radiation, the patient was still doing well seven years after diagnosis. The importance of electrophoretic studies of the cerebrospinal fluid protein and the serum protein is discussed. The literature on solitary intracranial plasmacytoma is reviewed.
A rare case of solitary plasmacytoma of the sphenoid sinus involving the pituitary fossa is reported. A 50-year-old woman with a history of diplopia and a mass in the sphenoid sinus and the sellar region, documented by computed tomography, was referred to our department with a presumed diagnosis of nonfunctioning pituitary adenoma. The clinical and biochemical characteristics were unrevealing, but magnetic resonance imaging examination demonstrated the extrapituitary origin of the lesion. The patient was operated on by the transsphenoidal approach, and the lesion was histologically diagnosed as a plasmacytoma. Review of the literature disclosed 11 previously described cases of myelomatous disease presenting clinically as a pituitary adenoma. Our case demonstrates that magnetic resonance imaging investigation may help in distinguishing the extrapituitary origin of a mass involving the pituitary fossa.
Plasmacytoma, a localized extramedullary mass of plasma cells, is one manifestation of multiple myeloma. Renal plasmacytomas are rare lesions with neovascularity on arteriogram. This may cause confusion with the much more common renal cell carcinoma unless the clinician is aware of this entity. A case is presented, the literature reviewed, and problems in management discussed.
A seventy-six-year-old woman with plasmacytoma presenting as a renal mass died three months after diagnosis. Bone surveys disclosed no lytic lesions. Gallium-67 scan showed an avid uptake of the radionuclide in the renal mass. Histologic and immunofluorescence studies of resected specimens demonstrated that the renal parenchyma was destroyed by sheets of immature plasma cells producing IgM-lambda and by massive deposits of amorphous, eosinophilic substance stained with anti-mu and anti-lambda antisera. The literature is reviewed. We believe this case is the first one of well-documented IgM-producing renal plasmacytoma.
The karyotypes of pristane-induced mouse plasmacytomas were studied by G banding. Only primary tumors or early passage generations were analyzed. In contrast to murine T cell leukemias that showed a regular trisomy of chromosome 15, all plasmacytomas showed a consistent translocation of the distal part of chromosome 15 to either chromosome 6 [rcpT(6;15)] or 12 [T(12;15)]. The specific breakpoints were at 6C, 15D3/E ro D2/3 and 12F2. Early passage generations often showed a mixed population with two different translocations, suggesting polyclonal origin. Considered together with the known karyotypic features of murine and human lymphomas, these findings support the theory that the nonrandom chromosomal changes in lymphoproliferative malignancies are associated with the type of the target cell, rather than with the etiological agent. Moreover, the involvement of the chromosomes known to carry the heavy chain (12) and the light chain (6) determinants, respectively, raises the question of whether the translocations may be related to the DNA level rearrangements known to occur during the differentiation of normal plasma cells.
The 15;12 translocations in murine plasmacytomas represent recombination of the myc oncogene with the immunoglobulin CH locus, often within CH switch recombination (SH) regions. Chromosome junctions cloned from four plasmacytomas confirmed that the translocation generates reciprocal CHSH-myc and 5'myc-SH structures. H locus targets included S alpha fused to Smu, Smu fused to S gamma 2b, and a germline S alpha region. The nature of two H locus targets suggests that the target need not be highly active transcriptionally. Switch recombination machinery is implicated in the translocation by the SH targets and by homology of certain c-myc breakpoints with normal switch recombination sites. Fusion regions revealed deletions, extraneous nucleotides, and one duplication. These results prompt a translocation model in which staggered single-stranded breaks on each chromosome are followed by single-strand excision or polymerization prior to ligation to the other chromosome.
Nucleoplasmic and non-histone chromatin proteins from two unrelated and four related mouse plasmacytoma cell lines have been analysed by biosynthetic labelling with [35S]-methionine followed by one- and two-dimensional polyacrylamide gel electrophoresis. We have attempted to find a relationship between the patterns of nuclear proteins and gene expression in mutant plasmacytoma cell lines. The majority of nuclear proteins are common to all of the cell lines studied as would be expected if the majority of nuclear proteins are concerned with functions common to all plasma cells. There are, however, both qualitative and quantitative differences in the nuclear protein patterns of mutant and parent cell lines which appear to correlate with differences in gene expression. The turnover of nuclear proteins in two of the cell lines, MOPC 315.40 (IgA producer) and MOPC 315.32 (lambda 2 chain producer) was studied using pulse-chase techniques.
The identification of enhancers at the 3' end of the IgH locus has prompted a re-evaluation of the regulation of Ig gene expression. Moreover, these elements may provide a possible explanation as to how the c-myc oncogene becomes dysregulated upon translocation into the IgH locus with the IgH intragenic enhancer on the reciprocal chromosome. These 3' enhancers have also been shown to redirect promoter utilization on c-myc reporter gene constructs in transient transfection experiments. This region of the locus also contains the B cell specific IgH 3' enhancer. This temporally regulated enhancer has been implicated in the mechanisms that control class switch recombination. Here we demonstrate that overexpression of the c-myc protein in mouse plasmacytoma cells (MPC-11) and HeLa cells can transcriptionally upregulate a reporter gene construct driven by a subregion (domain C) of the IgH 3' enhancer. Domain C contains a functional dual symmetry E-box motif, CACGTG. The DNA binding experiments demonstrate that USF was the major factor interacting with this motif. Based on these observations, we speculate that the c-myc protein may upregulate expression of translocated c-myc in mouse plasmacytomas possibly via an USF-binding E-box motif in the IgH 3' enhancer.
The effects of pristane on some immunity parameters in BALB/c mice were studied. The intraperitoneal administration of a single dose of pristane induced a strong inflammatory reaction that lasted longer than 10 days. When mice were immunized with sheep erythrocytes 10 days after pristane administration, the response of hemolytic IgM-forming cells was increased and that of hemolytic IgG-forming cells was decreased; however, the total number of antibody-forming cells did not change. The proliferative response of splenocytes to concanavalin A was increased in mice that received pristane 10 days earlier. Development of the syngeneic NS1 plasmacytoma was enhanced by administration of pristane 2 days or 10 days before tumor transplantation. We concluded that enhancement of plasmacytoma development was not due to immunosuppressive properties of pristane but to other factors such as ascites induction.
A review of 46 cases of solitary plasmacytoma of bone was undertaken in an attempt to better define the clinical features and prognostic indicators associated with this disease. Criteria for inclusion in the study included the following: (a) solitary lytic bone lesion on skeletal survey; (b) histologic confirmation of the lesion; and (c) bone marrow plasmacytosis of less than 10 percent. Patients with extramedullary plasmacytomas and osteosclerotic lesions were excluded. All patients were evaluated with serum and urine protein studies at the time of diagnosis. The median follow-up was 90 months with a minimum of 30 months. Fifty-four percent of the lesions involved the vertebral column. The thoracic spine was the single most commonly involved site (13/46 patients). The initial lesion was treated with radiotherapy in all but three patients in whom complete surgical resection was achieved. Total doses ranged from less than 20 Gy to 70 Gy with a median of 39.75 Gy. Overall, 54% developed multiple myeloma, 2% failed with new bone lesions without multiple myeloma, and 11% developed local recurrences. No patient receiving 45 Gy or more to the solitary lesion had a local failure. While the median time to progression was 18 months, 23% of the failures occurred after 60 months. The five local failures occurred at 7, 12, 18, 40, and 114 months. The overall survival was 74% at 5 years and 45% at 10 years. The 5- and 10-year disease-free survivals, however, were 43 and 25%, respectively. Evidence of abnormal serum and/or urine protein was found in 25 of 46 patients. Neither survival nor disease-free survival was significantly influenced by the presence of abnormal proteins even if they persisted after irradiation.
The chromosomal translocation associated with many tumors of immunoglobulin-producing cells frequently results in the joining of the immunoglobulin heavy-chain locus and the c-myc oncogene. This translocation of c-myc has profound structural and functional consequences for the oncogene, including loss of the 5' end of the gene and transcriptional deregulation. We report in this communication that translocation results in a new methylation pattern of c-myc. In normal kidney and liver tissue, the c-myc gene is methylated at its 3' end. The translocated gene in plasmacytoma DNA is extensively demethylated. On the other hand, the nonrearranged c-myc gene in plasmacytoma DNA (which is transcriptionally silent) is extensively methylated. In addition, we confirm the nucleotide sequence (with 19 discrepancies out of 1400 bp) 5' to the murine c-myc gene, as reported by Corcoran et al. [Cell 40 (1985) 71-79].
A patient with multiple myeloma developed neurological symptoms consistent with an expanding intracranial lesion. The CT scan showed evidence of a posterior fossa mass. This was pathologically confirmed to be a plasmacytoma. There was no CT or pathological evidence of bone involvement. When the neurological symptoms developed, there was no systemic evidence of recurrence of the myeloma. The intracranial manifestations of multiple myeloma are reviewed. The CT findings and differential diagnosis of the plasmacytoma are reviewed.
A case of extramedullary plasmacytoma of the larynx is presented. The case is unique for the extramedullary plasmacytoma's marked extension into the mediastinum. The computed tomography (CT) features are described.
Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm that occurs in the soft tissues, especially in the upper airways. We report two cases of laryngeal EMP, both of which were treated by surgical resection, one with and one without combination radiotherapy. A 39-year-old male was diagnosed with a laryngeal tumor localized in the left arytenoid region in October 1999. Biopsy showed the tumor to be a plasmacytoma. He underwent a direct laryngoscopy and resection of the tumor with a KTP laser. Consequently, he received radiotherapy to a total dose of 60 Gy. He has remained clear, with no local recurrence during 6 years of follow-up. A 59-year-old male was diagnosed with an epiglottic tumor in December 1989. He underwent a direct laryngoscopy and removal of the tumor. Histopathological study of the tumor showed a diffuse proliferation of plasmacytoid cells. He has remained diseasefree during 15 years of follow-up.
Apart from calvarial infiltration, intracranial involvement in multiple myeloma is uncommon. Diffuse leptomeningeal invasion with or without parenchymal involvement is most common. Dural infiltration without involvement of the parenchyma, leptomeninges or skull is rare. The differential diagnosis of a dural plasmacytoma includes meningioma, which has a similar MRI appearance, metastasis, lymphoma and sarcoma of the dura mater. We present a patient with multiple myeloma presenting with an intracerebral mass mimicking a meningioma on MRI. Multiple myeloma had been diagnosed seven years previously. The patient presented with headache and speech disturbance 12 months after autologous peripheral stem cell transplantation for recurrence of multiple myeloma. MRI revealed a left temporal extra-axial mass with a dural tail mimicking meningioma. Histopathological examination of the mass after excision showed multiple myeloma immunopositive for IgG, kappa light chain and CD38. There was no recurrence after postoperative radiotherapy. Plasmacytoma should be considered in the differential diagnosis of a solitary dural mass, particularly in a patient with multiple myeloma.
A 71-year-old woman was referred to our hospital because of hyperproteinemia and serum M-protein (IgG-lambda type). Chest computed tomographic (CT) scan revealed a tumor in each lung and transbronchial lung biopsy was performed. Histopathological examination showed monotonous medullary proliferation of morphologically mature plasma cells. These cells were cIgG+, cIg-lambda+, CD 20+, CD 79 a+, CD 138+, cIg-kappa-, and CD3-. Since there were very few non-neoplastic plasma cells and small lymphocytes in addition to the absence of reactive lymph follicles and fibrosis, the patient was diagnosed as having plasmacytoma. There was no proliferation of plasma cells in the bone marrow. Thus, the lesion was finally characterized as primary pulmonary plasmacytoma. Treatment with melphalan/prednisolone resulted in considerable decrease in the serum IgG level and regression of the pulmonary tumors. The effectiveness of the chemotherapy could confirm our diagnosis, although MALT-type lymphoma with plasmacytic differentiation cannot be completely ruled out.
This is the case report of a 45-year-old lady presenting with headache and papilledema. Investigations revealed falcotentorial plasmacytoma. Tumor was removed and adjuvant radiotherapy was given. This is curative. There are only 2 previous cases of plasmacytoma involving falx and tentorium simultaneously. En plaque meningioma is the close differential diagnosis.
BACKGROUND: Multiple myeloma (MM) presentation with cerebral mass lesion is unusual. Gamma knife radiosurgery for plasmacytoma has not been reported so far. CASE REPORT: We report a 70-year-old female with a medical history of infiltrative ductal carcinoma of the breast. She developed cavernous sinus syndrome (CSS) 5 months before admission to the hospital. The magnetic resonance imaging revealed an isointense solitary mass in the left cavernous sinus in noncontrast T1-weighted images. The lesion was highly enhancing with gadolinium-diethylenetriaminopentaacetic acid. She was operated by using Dolenc technique, and the tumor was partially resected. The pathological examination of the tumor tissue revealed a plasmacytoma. Systemic evaluation was positive for the diagnosis of MM. She underwent gamma knife radiosurgery for the residual cavernous sinus tumor. Chemotherapy with prednisolone and melphalan was given. Follow-up magnetic resonance images 6 months after the treatment demonstrated complete tumor disappearance. However, she died of sepsis 26 months after the diagnosis. CONCLUSION: This is an unusual MM case with a history of breast cancer, which had CSS and which demonstrated an excellent response to gamma knife radiosurgery.